Orbit, 2014; 33(4): 311–313 ! Informa Healthcare USA, Inc. ISSN: 0167-6830 print / 1744-5108 online DOI: 10.3109/01676830.2014.904376
C ASE REPORT
Allergic Fungal Sinusitis Involving the Lacrimal Sac: A Case Report and Review Kristina Y. Pao1, Vladimir Yakopson2, Joseph C. Flanagan2, and Ralph C. Eagle, Jr.3
Orbit Downloaded from informahealthcare.com by Memorial University of Newfoundland on 01/21/15 For personal use only.
1
Wills Eye Institute at Thomas Jefferson University, Philadelphia, PA, USA, 2Oculoplastic and Orbital Surgery Service. Wills Eye Institute, Philadelphia, PA, USA, and 3Ocular Pathology Service, Wills Eye Institute, Philadelphia, PA, USA
ABSTRACT Background: We report a case of allergic fungal sinusitis (AFS) involving the lacrimal sac and review the current English literature. A literature search for AFS involving the lacrimal sac revealed two reports with only one of the two cases demonstrating histological evidence of fungal elements. This is just the third such case and only the second reported case with histopathologic confirmation of fungal elements by Gomori methenamine silver (GMS) stain. Materials and Methods: A PubMed database search was performed using combinations of the following key words: allergic fungal sinusitis, lacrimal sac, nasolacrimal duct, ophthalmology, epiphora, orbit. A 70-year-old white man with a history of chronic conjunctivitis and nasal polyps presenting with chronic epiphora was found to have dacryostenosis on the left side. A CT scan of the orbits revealed mucoperiosteal thickening completely obliterating the frontal, ethmoid and sphenoid sinuses. Results: A left external dacryocystorhinostomy (DCR) was performed and the lacrimal sac contents were studied histopathologically. Microscopic examination of the lacrimal sac contents disclosed allergic mucin with laminated aggregates of eosinophils in various stages of degeneration, Charcot–Leyden crystals and rare noninvasive fungal hyphae confirming the diagnosis of AFS. Fungal elements stained positively with Gomori methenamine silver stain. Conclusion: Although rarely reported, AFS can affect the lacrimal sac. AFS should be suspected in patients with a history of recurrent refractory sinusitis, recurrent dacryocystitis and nasal polyposis. Early diagnosis is important for adequate treatment and prevention of recurrence. Keywords: Allergic fungal sinusitis, lacrimal sac, nasolacrimal duct
INTRODUCTION
histopathologic confirmation of fungal elements reported in only one of the two published cases.1,10 Here, we present a third case of lacrimal sac involvement and the second case demonstrating representative pathologic findings confirming the presence of fungal elements within the lacrimal sac contents.
Allergic fungal sinusitis (AFS) has a prevalence of 6.5% and often causes a recurrent sinusitis in immunocompetent patients unresponsive to therapy.1 AFS has been described in the ophthalmic literature affecting the orbit causing unilateral or bilateral proptosis, blepharoptosis, epiphora, ophthalmoplegia, facial deformity, asthenopia, diplopia, orbital abscess formation, visual loss, optic neuropathy and intracranial extension.2–10 However, lacrimal sac involvement by this condition has only been reported twice with
MATERIALS AND METHODS The patient’s history, examination and outcome were reviewed retrospectively. A thorough literature search
Received 20 September 2013; Revised 27 February 2014; Accepted 10 March 2014; Published online 14 May 2014 Correspondence: Kristina Y. Pao M.D., 1609 Woodbourne Road #303, Levittown, PA, 19057, Tel: 215-547-1818, Fax: 215-547-5174, E-mail: [email protected]
311
312 K. Pao et al. of English-language publications was completed on the PubMed database using key words: allergic fungal sinusitis, lacrimal sac, nasolacrimal duct, ophthalmology, epiphora, orbit. Two published case reports of allergic fungal sinusitis involving the lacrimal sac were found.
Orbit Downloaded from informahealthcare.com by Memorial University of Newfoundland on 01/21/15 For personal use only.
CASE REPORT/RESULTS A 70-year-old white male presented with tearing from the left eye for 6 weeks. The patient noted that a ‘‘bump’’ developed overlying his left lacrimal sac one-month prior. He reported no history of discharge, pain or trauma. The patient’s past medical history was significant for seasonal allergies, nasal polyps and right nasolacrimal duct obstruction. The patient’s past surgical history was significant for an uncomplicated right external DCR with Crawford silicone stent placement and an uncomplicated nasal polypectomy 3 years prior to presentation. Examination revealed a corrected visual acuity of 20/25 and 20/30 in the right and left eyes, respectively. A firm, sub-centimeter mass was palpated inferior to the left medical canthus that failed to demonstrate reflux with pressure. Probing and irrigation of the dilated left lower punctum showed 100% reflux. Slit lamp biomicroscopy was significant for an increased amount of mucus in the tear film of the left eye. The remainder of the exam was unremarkable. A CT scan of the orbits revealed mucoperiosteal thickening completely obliterating the frontal, ethmoid and sphenoid sinuses. No lacrimal sac tumor was identified or suspected. The patient underwent elective left external DCR with placement of Crawford silicone tubes. Histopathologic examination of the contents of the lacrimal sac disclosed sheets of amphophilic allergic mucin containing laminated aggregates of eosinophils in various stages of degeneration, multiple CharcotLeyden crystals and fungal hyphae confirmed by GMS stain (Figure 1). The fungi were found within the mucin and did not invade the surrounding tissue. The findings were consistent with allergic fungal sinusitis. Fungal cultures were not obtained.
DISCUSSION A thorough literature search was performed to assess whether previously reported cases of AFS involving the lacrimal sac existed. Our search yielded two reported cases of AFS involving the lacrimal sac, both reported by otolaryngology. Facer and colleagues reported the first case of AFS involving the lacrimal
FIGURE 1. Histopathology of left lacrimal sac contents. Main figure (Hematoxylin-eosin 50) shows allergic mucin consistent with allergic fungal sinusitis aggregates of degenerated eosinophils. Inset (Gomori methenamine silver stain for fungus, 250) demonstrates noninvasive fungal hyphae within mucin.
sac in 2003 in a 69-year-old male with a history of nasal polyps and prior sinus surgery presenting with a cystic lesion in the right medial canthal region.1 Histopathologic examination of surgical specimens obtained from the patient’s endoscopic DCR revealed findings consistent with AFS with positive staining for fungal hyphae on Chitinase immunofluorescein stains. Kim et al. published the second case report of AFS involving the lacrimal sac in 2013 in a 54-year-old female with a history of asthma and nasal polyps presenting with left dacryocystitis unresponsive to topical and systemic antibiotics.10 Histopathologic examination of the lacrimal sac revealed marked eosinophilia ‘‘highly suggestive of allergic fungal sinusitis’’.10 However, GMS stain for fungal elements was negative. In 2011, Petkar and associates reported a case of AFS with massive intracranial extension in a 24-year-old male presenting with epiphora.7 Radiographic imaging demonstrated a mass with fluid involving the nasopharynx, paranasal sinuses, left frontal sinus extending intracranially compressing the frontal lobe. AFS was confirmed on histologic exam with fungal elements on GMS stain; however, the authors did not report if the lacrimal sac was biopsied. The presentation of AFS and lacrimal sac tumors are similar and may cause unilateral findings such as epiphora, proptosis, blepharoptosis, ophthalmoplegia, recurrent dacryocystitis and a firm mass in the medial canthal area. However, AFS patient typically lack bloody discharge and lymphadenopathy and rarely present with a firm mass that extends superior to the medial canthal region. AFS patients typically have a history of chronic rhinosinusitis, allergic rhinitis atopy Orbit
Orbit Downloaded from informahealthcare.com by Memorial University of Newfoundland on 01/21/15 For personal use only.
Allergic Fungal Sinusitis of the Lacrimal Sac 313 and nasal polyps. Facer and colleagues postulate that distal obstruction, often due to polyposis, and distension of the lacrimal sac leads to chronic reflux of eosinophilic mucin into the nasolacrimal system.1 Other predisposing factors include nasal tumors, septal deviations, prior nasal surgery, foreign bodies, cystic fibrosis, asthma, atopy and ciliary defects.11 Our patient had a history of chronic sinusitis, nasal polyposis and history of prior nasal and lacrimal surgery. Imaging in AFS typically reveals a soft tissue lesion with heterogenous opacification of the sinuses on CT scan and isointense or slightly hyperintense signal on T1-weighted MRI and hypointense signal on T2-weighted MRI.6 Imaging of our patient revealed extensive sinus disease but no frank lesion. Treatment of AFS includes topical and systemic steroids, surgical debridement and aeration of involved sinuses. Systemic antifungals have not been shown to be efficacious in the treatment of AFS.12 Since his surgery, our patient has shown no evidence of recurrence of nasolacrimal duct obstruction.
CONCLUSION AFS should be suspected in patients with a history of recurrent refractory sinusitis, recurrent dacryocystitis and nasal polyps. Rarely, AFS may involve the lacrimal sac prompting the patient to present to the ophthalmologist. Diagnosis is paramount for adequate treatment and prevention of recurrence. We describe the third reported case of AFS involving the lacrimal sac and the second case with histopathologic confirmation of fungal elements within the lacrimal sac by GMS stain.
!
2014 Informa Healthcare USA, Inc.
DECLARATION OF INTEREST The authors report no conflicts of interest. The authors alone are responsible for the content and writing of the paper.
REFERENCES 1. Facer ML, Ponikau JU, Sherris DA. Eosinophilic fungal rhinosinusitis of the lacrimal sac. Laryngoscope 2003;113: 210–4. 2. Klapper SR, Lee AG, Patrinely JR, et al. Orbital involvement in allergic fungal sinusitis. Ophthalmol 1997;4: 2094–2100. 3. Geren BN, Brown HH, Hearnsberger 3rd HG, Westfall CT. Allergic fungal sinusitis with unilateral eye involvement. Arch Ophthalmol 2004;122:1390–1393. 4. Chang W, Shields CL, Shields JA, et al. Bilateral orbital involvement with massive allergic fungal sinusitis. Arch Ophthalmol 1996;114:767–768. 5. Kurimoto T, Tonari M, Ishizaki N, et al. A case of eosinophilic chronic rhinosinusitis associated with optic neuropathy. Clin Ophthalmol 2011;5:853–856. 6. Chang WJ, Tse DT, Bressler KL, et al. Diagnosis and management of allergic fungal sinusitis with orbital involvement. Ophthal Plast Reconstr Surg 2000;16:72–74. 7. Petkar A, Rao L, Elizondo DR, et al. Allergic fungal sinusitis with massive intracranial extension presenting with tearing. Ophthal Plast Reconstr Surg 2011;27:98–100. 8. Alsagoob AA, Taguri AH, Al-Ahmary AY, Sari LM. Asthenopia as the presenting symptom in advance allergic fungal sinusitis. Ophthalmol 2012;26:339–341. 9. Carter KD, Graham SM, Carpenter KM. Ophthalmic manifestations of allergic fungal sinusitis. Am J Ophthalmol 1999;127:189–195. 10. Kim C, Kacker A, Chee RI, Lelli GJ. Allergic fungal sinusitis causing nasolacrimal duct obstruction. Orbit 2013; 32:143–145. 11. Goldstein MF. Allergic fungal sinusitis: an underdiagnosed problem. Hosp Pract 1992;27:73–74. 12. Marple BF. Allergic fungal rhinosinusitis: current theories and management strategies. Largyngoscope 2001;111: 1006–1019.
C ASE REPORT
Allergic Fungal Sinusitis Involving the Lacrimal Sac: A Case Report and Review Kristina Y. Pao1, Vladimir Yakopson2, Joseph C. Flanagan2, and Ralph C. Eagle, Jr.3
Orbit Downloaded from informahealthcare.com by Memorial University of Newfoundland on 01/21/15 For personal use only.
1
Wills Eye Institute at Thomas Jefferson University, Philadelphia, PA, USA, 2Oculoplastic and Orbital Surgery Service. Wills Eye Institute, Philadelphia, PA, USA, and 3Ocular Pathology Service, Wills Eye Institute, Philadelphia, PA, USA
ABSTRACT Background: We report a case of allergic fungal sinusitis (AFS) involving the lacrimal sac and review the current English literature. A literature search for AFS involving the lacrimal sac revealed two reports with only one of the two cases demonstrating histological evidence of fungal elements. This is just the third such case and only the second reported case with histopathologic confirmation of fungal elements by Gomori methenamine silver (GMS) stain. Materials and Methods: A PubMed database search was performed using combinations of the following key words: allergic fungal sinusitis, lacrimal sac, nasolacrimal duct, ophthalmology, epiphora, orbit. A 70-year-old white man with a history of chronic conjunctivitis and nasal polyps presenting with chronic epiphora was found to have dacryostenosis on the left side. A CT scan of the orbits revealed mucoperiosteal thickening completely obliterating the frontal, ethmoid and sphenoid sinuses. Results: A left external dacryocystorhinostomy (DCR) was performed and the lacrimal sac contents were studied histopathologically. Microscopic examination of the lacrimal sac contents disclosed allergic mucin with laminated aggregates of eosinophils in various stages of degeneration, Charcot–Leyden crystals and rare noninvasive fungal hyphae confirming the diagnosis of AFS. Fungal elements stained positively with Gomori methenamine silver stain. Conclusion: Although rarely reported, AFS can affect the lacrimal sac. AFS should be suspected in patients with a history of recurrent refractory sinusitis, recurrent dacryocystitis and nasal polyposis. Early diagnosis is important for adequate treatment and prevention of recurrence. Keywords: Allergic fungal sinusitis, lacrimal sac, nasolacrimal duct
INTRODUCTION
histopathologic confirmation of fungal elements reported in only one of the two published cases.1,10 Here, we present a third case of lacrimal sac involvement and the second case demonstrating representative pathologic findings confirming the presence of fungal elements within the lacrimal sac contents.
Allergic fungal sinusitis (AFS) has a prevalence of 6.5% and often causes a recurrent sinusitis in immunocompetent patients unresponsive to therapy.1 AFS has been described in the ophthalmic literature affecting the orbit causing unilateral or bilateral proptosis, blepharoptosis, epiphora, ophthalmoplegia, facial deformity, asthenopia, diplopia, orbital abscess formation, visual loss, optic neuropathy and intracranial extension.2–10 However, lacrimal sac involvement by this condition has only been reported twice with
MATERIALS AND METHODS The patient’s history, examination and outcome were reviewed retrospectively. A thorough literature search
Received 20 September 2013; Revised 27 February 2014; Accepted 10 March 2014; Published online 14 May 2014 Correspondence: Kristina Y. Pao M.D., 1609 Woodbourne Road #303, Levittown, PA, 19057, Tel: 215-547-1818, Fax: 215-547-5174, E-mail: [email protected]
311
312 K. Pao et al. of English-language publications was completed on the PubMed database using key words: allergic fungal sinusitis, lacrimal sac, nasolacrimal duct, ophthalmology, epiphora, orbit. Two published case reports of allergic fungal sinusitis involving the lacrimal sac were found.
Orbit Downloaded from informahealthcare.com by Memorial University of Newfoundland on 01/21/15 For personal use only.
CASE REPORT/RESULTS A 70-year-old white male presented with tearing from the left eye for 6 weeks. The patient noted that a ‘‘bump’’ developed overlying his left lacrimal sac one-month prior. He reported no history of discharge, pain or trauma. The patient’s past medical history was significant for seasonal allergies, nasal polyps and right nasolacrimal duct obstruction. The patient’s past surgical history was significant for an uncomplicated right external DCR with Crawford silicone stent placement and an uncomplicated nasal polypectomy 3 years prior to presentation. Examination revealed a corrected visual acuity of 20/25 and 20/30 in the right and left eyes, respectively. A firm, sub-centimeter mass was palpated inferior to the left medical canthus that failed to demonstrate reflux with pressure. Probing and irrigation of the dilated left lower punctum showed 100% reflux. Slit lamp biomicroscopy was significant for an increased amount of mucus in the tear film of the left eye. The remainder of the exam was unremarkable. A CT scan of the orbits revealed mucoperiosteal thickening completely obliterating the frontal, ethmoid and sphenoid sinuses. No lacrimal sac tumor was identified or suspected. The patient underwent elective left external DCR with placement of Crawford silicone tubes. Histopathologic examination of the contents of the lacrimal sac disclosed sheets of amphophilic allergic mucin containing laminated aggregates of eosinophils in various stages of degeneration, multiple CharcotLeyden crystals and fungal hyphae confirmed by GMS stain (Figure 1). The fungi were found within the mucin and did not invade the surrounding tissue. The findings were consistent with allergic fungal sinusitis. Fungal cultures were not obtained.
DISCUSSION A thorough literature search was performed to assess whether previously reported cases of AFS involving the lacrimal sac existed. Our search yielded two reported cases of AFS involving the lacrimal sac, both reported by otolaryngology. Facer and colleagues reported the first case of AFS involving the lacrimal
FIGURE 1. Histopathology of left lacrimal sac contents. Main figure (Hematoxylin-eosin 50) shows allergic mucin consistent with allergic fungal sinusitis aggregates of degenerated eosinophils. Inset (Gomori methenamine silver stain for fungus, 250) demonstrates noninvasive fungal hyphae within mucin.
sac in 2003 in a 69-year-old male with a history of nasal polyps and prior sinus surgery presenting with a cystic lesion in the right medial canthal region.1 Histopathologic examination of surgical specimens obtained from the patient’s endoscopic DCR revealed findings consistent with AFS with positive staining for fungal hyphae on Chitinase immunofluorescein stains. Kim et al. published the second case report of AFS involving the lacrimal sac in 2013 in a 54-year-old female with a history of asthma and nasal polyps presenting with left dacryocystitis unresponsive to topical and systemic antibiotics.10 Histopathologic examination of the lacrimal sac revealed marked eosinophilia ‘‘highly suggestive of allergic fungal sinusitis’’.10 However, GMS stain for fungal elements was negative. In 2011, Petkar and associates reported a case of AFS with massive intracranial extension in a 24-year-old male presenting with epiphora.7 Radiographic imaging demonstrated a mass with fluid involving the nasopharynx, paranasal sinuses, left frontal sinus extending intracranially compressing the frontal lobe. AFS was confirmed on histologic exam with fungal elements on GMS stain; however, the authors did not report if the lacrimal sac was biopsied. The presentation of AFS and lacrimal sac tumors are similar and may cause unilateral findings such as epiphora, proptosis, blepharoptosis, ophthalmoplegia, recurrent dacryocystitis and a firm mass in the medial canthal area. However, AFS patient typically lack bloody discharge and lymphadenopathy and rarely present with a firm mass that extends superior to the medial canthal region. AFS patients typically have a history of chronic rhinosinusitis, allergic rhinitis atopy Orbit
Orbit Downloaded from informahealthcare.com by Memorial University of Newfoundland on 01/21/15 For personal use only.
Allergic Fungal Sinusitis of the Lacrimal Sac 313 and nasal polyps. Facer and colleagues postulate that distal obstruction, often due to polyposis, and distension of the lacrimal sac leads to chronic reflux of eosinophilic mucin into the nasolacrimal system.1 Other predisposing factors include nasal tumors, septal deviations, prior nasal surgery, foreign bodies, cystic fibrosis, asthma, atopy and ciliary defects.11 Our patient had a history of chronic sinusitis, nasal polyposis and history of prior nasal and lacrimal surgery. Imaging in AFS typically reveals a soft tissue lesion with heterogenous opacification of the sinuses on CT scan and isointense or slightly hyperintense signal on T1-weighted MRI and hypointense signal on T2-weighted MRI.6 Imaging of our patient revealed extensive sinus disease but no frank lesion. Treatment of AFS includes topical and systemic steroids, surgical debridement and aeration of involved sinuses. Systemic antifungals have not been shown to be efficacious in the treatment of AFS.12 Since his surgery, our patient has shown no evidence of recurrence of nasolacrimal duct obstruction.
CONCLUSION AFS should be suspected in patients with a history of recurrent refractory sinusitis, recurrent dacryocystitis and nasal polyps. Rarely, AFS may involve the lacrimal sac prompting the patient to present to the ophthalmologist. Diagnosis is paramount for adequate treatment and prevention of recurrence. We describe the third reported case of AFS involving the lacrimal sac and the second case with histopathologic confirmation of fungal elements within the lacrimal sac by GMS stain.
!
2014 Informa Healthcare USA, Inc.
DECLARATION OF INTEREST The authors report no conflicts of interest. The authors alone are responsible for the content and writing of the paper.
REFERENCES 1. Facer ML, Ponikau JU, Sherris DA. Eosinophilic fungal rhinosinusitis of the lacrimal sac. Laryngoscope 2003;113: 210–4. 2. Klapper SR, Lee AG, Patrinely JR, et al. Orbital involvement in allergic fungal sinusitis. Ophthalmol 1997;4: 2094–2100. 3. Geren BN, Brown HH, Hearnsberger 3rd HG, Westfall CT. Allergic fungal sinusitis with unilateral eye involvement. Arch Ophthalmol 2004;122:1390–1393. 4. Chang W, Shields CL, Shields JA, et al. Bilateral orbital involvement with massive allergic fungal sinusitis. Arch Ophthalmol 1996;114:767–768. 5. Kurimoto T, Tonari M, Ishizaki N, et al. A case of eosinophilic chronic rhinosinusitis associated with optic neuropathy. Clin Ophthalmol 2011;5:853–856. 6. Chang WJ, Tse DT, Bressler KL, et al. Diagnosis and management of allergic fungal sinusitis with orbital involvement. Ophthal Plast Reconstr Surg 2000;16:72–74. 7. Petkar A, Rao L, Elizondo DR, et al. Allergic fungal sinusitis with massive intracranial extension presenting with tearing. Ophthal Plast Reconstr Surg 2011;27:98–100. 8. Alsagoob AA, Taguri AH, Al-Ahmary AY, Sari LM. Asthenopia as the presenting symptom in advance allergic fungal sinusitis. Ophthalmol 2012;26:339–341. 9. Carter KD, Graham SM, Carpenter KM. Ophthalmic manifestations of allergic fungal sinusitis. Am J Ophthalmol 1999;127:189–195. 10. Kim C, Kacker A, Chee RI, Lelli GJ. Allergic fungal sinusitis causing nasolacrimal duct obstruction. Orbit 2013; 32:143–145. 11. Goldstein MF. Allergic fungal sinusitis: an underdiagnosed problem. Hosp Pract 1992;27:73–74. 12. Marple BF. Allergic fungal rhinosinusitis: current theories and management strategies. Largyngoscope 2001;111: 1006–1019.
